了解自身免疫淋巴增生综合征和自身免疫淋巴增殖综合征样疾病的免疫失调表现谱。

IF 2.6 4区 医学 Q2 RHEUMATOLOGY Rheumatic Disease Clinics of North America Pub Date : 2023-08-23 DOI:10.1016/j.rdc.2023.07.001
Christopher Failing MD , Jennifer R. Blase MD, PhD , Kelly Walkovich MD
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引用次数: 0

摘要

作为一种免疫失调的疾病,自身免疫性淋巴增殖综合征(ALPS)源于第一个细胞凋亡信号介导的细胞凋亡(Fas)和Fas配体途径中的致病性变体,导致CD3+TCRαβ+CD4-CD8-T细胞的升高以及慢性淋巴增殖,恶性肿瘤的风险增加,对风湿病学家来说更重要,自身免疫风险增加。虽然免疫细胞减少是最常见的自身免疫现象,但人们越来越重视眼部、肌肉骨骼、肺部和肾脏的炎症表现,类似于更常见的风湿病。此外,通过基因检测,具有相似临床特征和靶向治疗机会的ALPS样疾病越来越得到认可,这突出了风湿病学家在识别和诊断这一系列疾病方面的需要。这篇综述将重点关注ALPS和ALPS样疾病的临床和实验室特征,旨在为风湿病学家了解病理生理驱动因素并区分诊断提供一个框架。
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Understanding the Spectrum of Immune Dysregulation Manifestations in Autoimmune Lymphoproliferative Syndrome and Autoimmune Lymphoproliferative Syndrome-like Disorders
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来源期刊
CiteScore
4.20
自引率
0.00%
发文量
84
审稿时长
>12 weeks
期刊介绍: Rheumatic Disease Clinics updates you on the latest trends in patient management, keeps you up to date on the newest advances, and provides a sound basis for choosing treatment options. Each issue (February, May, August, and November) focuses on a single topic in rheumatology and is presented under the direction of an experienced editor. Topics include osteoarthritis, rheumatoid arthritis, crystal diseases, MSK exam, disorders of the bone, immunodeficiency states, lupus, neuromuscular disease, pain disorders, scleroderma, vasculitis, pediatrics, therapeutics, treatment guidelines, and treatment modalities.
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Contents Forthcoming Issues Imaging in Rheumatology Copyright Contributors
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